Mössbauer studies of frataxin role in iron-sulfur cluster assembly and dysfunction-related disease

被引：0

作者：

Ricardo Garcia-Serres

Martin Clémancey

Jean-Louis Oddou

Annalisa Pastore

Emmanuel Lesuisse

Jean-Marc Latour

机构：

[1] Université Joseph Fourier,

[2] CNRS,undefined

[3] UMR5249,undefined

[4] Medical Research Council National Institute for Medical Research,undefined

[5] Laboratoire Mitochondries,undefined

[6] Métaux et Stress oxydant,undefined

[7] Institut Jacques Monod,undefined

[8] CNRS-Université Paris,undefined

[9] CEA,undefined

[10] iRTSV,undefined

[11] LCBM,undefined

来源：

Hyperfine Interactions | 2012年 / 206卷

关键词：

Mössbauer spectroscopy; Frataxin; Iron sulfur cluster; Iron accumulation;

D O I：

暂无

中图分类号：

学科分类号：

摘要：

Friedreich ataxia is a disease that is associated with defects in the gene coding for a small protein frataxin. Several different roles have been proposed for the protein, including iron chaperoning and iron storage. Mössbauer spectroscopy was used to probe these hypotheses. Iron accumulation in mutant mitochondria unable to assemble iron sulfur clusters proved to be insensitive to overexpression of frataxin, ruling out its potential involvement as an iron storage protein similar to ferritin. Rather, it was found that frataxin negatively regulates iron sulfur cluster assembly.

引用

页码：13 / 18

页数：5

共 42 条

[31] Multiple Sense and Antisense Promoters Contribute to the Regulated Expression of the isc-suf Operon for Iron-Sulfur Cluster Assembly in Rhodobacter
Nie, Xin
Remes, Bernhard
Klug, Gabriele
MICROORGANISMS, 2019, 7 (12)
[32] A possible role for iron-sulfur cluster N2 in proton translocation by the NADH: Ubiquinone oxidoreductase (Complex I)
Flemming, D
Stolpe, S
Schneider, D
Hellwig, P
Friedrich, T
JOURNAL OF MOLECULAR MICROBIOLOGY AND BIOTECHNOLOGY, 2005, 10 (2-4) : 208 - 222
[33] Metamorphic protein IscU alternates conformations in the course of its role as the scaffold protein for iron-sulfur cluster biosynthesis and delivery
Markley, John L.
Kim, Jin Hae
Dai, Ziqi
Bothe, Jameson R.
Cai, Kai
Frederick, Ronnie O.
Tonelli, Marco
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[34] Iron-sulfur cluster biosynthesis: characterization of IscU-IscS complex formation and a structural model for sulfide delivery to the [2Fe-2S] assembly site
Nuth, Manunya
Cowan, J. A.
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[35] Essential role of the iron-sulfur cluster binding domain of the primase regulatory subunit Pri2 in DNA replication initiation
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PROTEIN & CELL, 2015, 6 (03) : 194 - 210
[36] MicroRNA-210 Controls Mitochondrial Metabolism during Hypoxia by Repressing the Iron-Sulfur Cluster Assembly Proteins ISCU1/2
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Zhang, Ying-Yi
Hemann, Craig
Mahoney, Christopher E.
Zweier, Jay L.
Loscalzo, Joseph
CELL METABOLISM, 2009, 10 (04) : 273 - 284
[37] Expanding the phenotype of mitochondrial disease: Novel pathogenic variant in ISCA1 leading to instability of the iron-sulfur cluster in the protein
Lebigot, E.
Hully, M.
Amazit, L.
Gaignard, P.
Michel, T.
Rio, M.
Lombes, M.
Therond, P.
Boutron, A.
Golinelli-Cohen, M. P.
MITOCHONDRION, 2020, 52 : 75 - 82
[38] Copper exerts cytotoxicity through inhibition of iron-sulfur cluster biogenesis on ISCA1/ISCA2/ISCU assembly proteins
Du, Jing
Huang, Zhaoyang
Li, Yanchun
Ren, Xueying
Zhou, Chaoting
Liu, Ruolan
Zhang, Ping
Lei, Guojie
Lyu, Jianxin
Li, Jianghui
Tan, Guoqiang
FREE RADICAL BIOLOGY AND MEDICINE, 2023, 204 : 359 - 373
[39] Mossbauer study of 4-hydroxybutyryl-CoA dehydratase - Probing the role of an iron-sulfur cluster in an overall non-redox reaction
Muh, U
Buckel, W
Bill, E
EUROPEAN JOURNAL OF BIOCHEMISTRY, 1997, 248 (02): : 380 - 384
[40] The human YAE1-ORAOV1 complex of the cytosolic iron-sulfur protein assembly machinery binds a [4Fe-4S] cluster
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